ZEB2 (zinc finger E-box binding homeobox 2) encodes the Smad Interacting Protein 1, which is involved in the TGF-β/BMP/Smad signaling cascade (Babkina et al., 2016). ZEB2 mRNA is expressed during early embryogenesis in brain tissue, and is thought to play an important role in neural crest cell migration (Van de Putte et al., 2003) and in the regulation of corticogenesis (Seuntjens et al. 2009). Mutations in ZEB2 have been linked with epilepsy (EPICURE Consortium et al., 2012) and related disorders, such as Hirschsprung disease/Mowat-Wilson syndrome (MWS; (Cordelli et al., 2013)). MWS is caused by heterozygous mutations or deletions of ZEB2 and is characterized by epilepsy, moderate to severe intellectual disability, corpus callosum abnormalities and other congenital malformations. Recent studies (Cordelli et al., 2013) suggest that a distinct “electroclinical” phenotype, characterized by age-dependent EEG changes, can be recognized in most patients with MWS. While the mechanism underlying epilepsy in individuals with ZEB2 mutations is not well understood, studies by McKinsey and Van den Berghe (McKinsey et al., 2013; van den Berghe et al., 2013) show the influence of ZEB2 on the
